Idiopathic pulmonary fibrosis and systemic sclerosis-interstitial lung disease: features at admission to a tertiary pulmonology unit

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Background: Idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-interstitial lung disease (SSc-ILD) are chronic fibrotic disorders that, despite sharing common profibrotic pathways, display differences in epidemilogy and clinical presentation. Objective: To investigate features of IPF and SSc-ILD patients admitted to a tertiary pulmonology unit for a recent onset/worsening dyspnea. Methods: Fourty-one IPF and 39 SSc-ILD patients visited over 1 year were investigated for lung function and comorbidities frequently associated with both conditions. Results: As expected, SSc-ILD were predominantly females and younger (median 61 vs 73 years old, p<0.0001). IPF patients were more frequently smokers, walked a shorter distance at 6 minute walking test (346 mt vs 423, p=0.02), and more frequently desaturated under effort (17/27 vs 9/29, p=0.03). However, the 2 groups did not significantly differ in % of predicted forced vital capacity (89.24 in SSc-ILD vs 85.03 in IPF) and diffusing lung capacity for CO (51.15 in SSc-ILD vs 54.94 in IPF), and had a similar proportion of previous oxygen therapy (10/39 in SSc-ILD vs 17/41 in IPF). In regard of comorbidities, gastroesophageal reflux was more frequent in SSc-ILD (27/39 vs 13/41, p=0.02), while there was no difference in the proportion of secondary pulmonary hypertension (4/39 in SSc-ILD vs 3/41 in IPF) and history of cancer (2/39 in SSc-ILD vs 5/41 in IPF). Conclusion: Despite differences in epidemilogical and clinical features, patients with SSc-ILD and IPF share common functional features at presentation.

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